BACKGROUND: We report an interesting case of asymptomatic retinal involvement in an encephalopathic patient enabling early identification of Susac's syndrome.
CASE PRESENTATION: A 39-year-old Caucasian lady with hearing loss and encephalopathy was referred for ophthalmic assessment, including screening for branch retinal artery occlusions characteristic of Susac's syndrome. Clinical features included severe headaches, right-sided hypoacusis, dysphasia and poor memory. Routine blood tests were normal. MRI brain showed numerous hyperintense lesions mainly in corpus callosum. Although she was visually asymptomatic, dilated funduscopy detected bilateral multiple peripheral branch retinal artery occlusions which were confirmed on fluorescein angiography. She was subsequently started on intravenous steroids and pulsed cyclophosphamide which improved her symptoms within 48 hours. Full recovery was made with no new arterial occlusions on four months follow-up.
CONCLUSION: The case further establishes the crucial role of a detailed ophthalmic examination supported by fluorescein angiography in the assessment of these patients, who are at risk of being misdiagnosed and undertreated.
- Corpus Callosum/pathology
- Cyclophosphamide/therapeutic use
- Drug Therapy, Combination
- Fluorescein Angiography
- Hearing Loss, Central/etiology
- Immunosuppressive Agents/therapeutic use
- Magnetic Resonance Imaging
- Memory Disorders/etiology
- Methylprednisolone/therapeutic use
- Retinal Artery Occlusion/diagnosis
- Susac Syndrome/complications